The current consensus for the diagnostic and treatment of extramammary Paget’s disease

Abstract

Extramammary Paget's disease is a slow-growing cutaneous intraepithelial adenocarcinoma of the apocrine glands in the anogenital and axillary regions. The disease is extremely rare, affects predominantly postmenopausal women, and has nonspecific clinical caracteristics, so that it can take 210 years from the first clinical signs to diagnosis. The vulva and the perianal region are the most affected zones. Extramammary Pagets disease is clinically manifested as well-defined erythematous plaques with secondary changes, such as scaling, ulceration and even bleeding. Differential diagnosis is made between primary and secondary forms of Extramammary Pagets disease, candidiasis, contact dermatitis, Crohn's disease, eczema, erosive lichen planus, hydradenitis suppurativa, Langerhans cell histiocytosis, sclerosing lichen, psoriasis, squamous cell carcinoma in situ, amelanotic melanoma and mycosis fungoide. Treatment mainly includes the surgical component, as well as the use of photodynamic therapy, cytostatic agents, and, depending on the stage, systemic chemotherapy.